SELECTED ARTICLES FROM THE RECENT LITERATURE 2006

10/16/06

Clinical profiles in hereditary angioedema

Summary
Background – Hereditary angioedema (HAE) is an unusual disorder that has achieved prominence because there is commonly prominent morbidity and a potential for sudden death due to airway obstruction.

Findings – Burk et al of Johannes Gutenberg Univ in Mainz, Germany reviewed some clinical aspects of 131,110 acute swelling episodes in 221 Patients with well-documented HAE followed by their group. These HAE episodes started at a mean age of 11.2 years with only 370 symptom free years (6.5%) out of 5,736 year cumulative duration of disease in this patient group. Swelling in the skin (extremity, facial, genital, trunk) and/or abdominal pain attacks characterized over 97% of the HAE acute episodes. In contrast, mucosal swellings were much less common, involving the larynx in 0.9%, soft palate in 0.6%, tongue in 0.3% Involvement of other internal organs other than the G-I tract (leading to abdominal pain) was even less common.

The course of HAE tended to be more severe in women and in those with onset of episodes earlier in life.

Reference
Am J Med 2006;119:267-74

Editor's Comments
This information is of interest and clinical utility because of the large patient group and relatively long duration of HAE in some patients (though much of the data was based on retrospective patient recall). I was surprised by the low frequency of mucosal involvement in the HAE episodes. I wonder whether treatment of at least some patients with altered androgens may have affected this pattern of tissue involvement.

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